Universität zu Köln

Goncalves, Ines do Carmo G., Rehorst, Wiebke A. and Kye, Min Jeong ORCID: 0000-0002-1323-7256 (2016). Dysregulation of RNA Mediated Gene Expression in Motor Neuron Diseases. CNS Neurol. Disord.-Drug Targets, 15 (8). S. 887 - 896. BUSUM: BENTHAM SCIENCE PUBL. ISSN 1996-3181

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Abstract

Recent findings indicate an important role for RNA-mediated gene expression in motor neuron diseases, including ALS (amyotrophic lateral sclerosis) and SMA (spinal muscular atrophy). ALS, also known as Lou Gehrig's disease, is an adult-onset progressive neurodegenerative disorder, whereby SMA or children's Lou Gehrig's disease is considered a pediatric neurodevelopmental disorder. Despite the difference in genetic causes, both ALS and SMA share common phenotypes; dysfunction/loss of motor neurons that eventually leads to muscle weakness and atrophy. With advanced techniques in molecular genetics and cell biology, current data suggest that these two distinct motor neuron diseases share more than phenotypes; ALS and SMA have similar cellular pathological mechanisms including mitochondrial dysfunction, oxidative stress and dysregulation in RNA-mediated gene expression. Here, we will discuss the current findings on these two diseases with specific focus on RNA-mediated gene regulation including miRNA expression, pre-mRNA processing and RNA binding proteins.

Item Type:	Journal Article
Creators:	Creators Email ORCID ORCID Put Code Goncalves, Ines do Carmo G. UNSPECIFIED UNSPECIFIED UNSPECIFIED Rehorst, Wiebke A. UNSPECIFIED UNSPECIFIED UNSPECIFIED Kye, Min Jeong UNSPECIFIED orcid.org/0000-0002-1323-7256 UNSPECIFIED
URN:	urn:nbn:de:hbz:38-288802
DOI:	10.2174/1871527315666160815164808
Journal or Publication Title:	CNS Neurol. Disord.-Drug Targets
Volume:	15
Number:	8
Page Range:	S. 887 - 896
Date:	2016
Publisher:	BENTHAM SCIENCE PUBL
Place of Publication:	BUSUM
ISSN:	1996-3181
Language:	English
Faculty:	Unspecified
Divisions:	Unspecified
Subjects:	no entry
Uncontrolled Keywords:	Keywords Language SPINAL MUSCULAR-ATROPHY; AMYOTROPHIC-LATERAL-SCLEROSIS; BINDING PROTEIN HUD; FRONTOTEMPORAL LOBAR DEGENERATION; MENTAL-RETARDATION PROTEIN; EMBRYONIC STEM-CELLS; KAPPA-B PATHWAY; MESSENGER-RNA; SMN COMPLEX; HUNTINGTONS-DISEASE Multiple languages Neurosciences; Pharmacology & Pharmacy Multiple languages
Refereed:	Yes
URI:	http://kups.ub.uni-koeln.de/id/eprint/28880